Livedo reticularis

Livedo reticularis

Description, Causes and Risk Factors:

ICD-10: R23.1.

Abbreviation: LR.

Alternative Name: Dermatopathia pigmentosa reticularis.

Livedo reticularis is a common dermatological finding. It is a vascular condition. It appears as a lace like purplish reticular pattern on the skin. It is usually seen on the lower extremities, and may be exacerbated be cold exposure.

It is probably caused due to swelling of the medium sized veins in the lower extremities. It may be a normal finding or may be associated with diseases associated with the blood, endocrine system, cardiovascular and autoimmune disorders like lupus, antiphospholipid antibody syndrome (APAS) or Sneddon's syndrome. It can also be connected with cardiovascular disease and certain cancers. In those instances, the livedo reticularis is secondary to the disorder causing it. It can also be a reaction to certain medications.

Types May Include:

Physiologic Livedo Reticularis (Cutis Marmorata): Physiologic livedo reticularis typically affects preterm infants, neonates, and fair-skinned females. The term “cutis marmorata” literally means “marbled skin.” Although it can be diffuse, it most often affects the lower extremities. The reticular discoloration occurs upon exposure to cold, yet ultimately completely dissipates with external warming. Although physiologic livedo reticularis usually spontaneously resolves, it can complicate patients with Down syndrome, Trisomy 18, Cornelia de Lange's syndrome, and Adams-Oliver syndrome throughout their lifetime.

Primary Livedo Reticularis: Primary livedo reticularis has a waxing and waning appearance. However, it differs from Physiologic livedo reticularis as its color changes are temperature independent. Interestingly, limb elevation reduces the discoloration in Primary livedo reticularis. Pathophysiologically, primary livedo reticularis is thought to be related to spontaneous arteriolar vasoconstriction with subsequent dermal hypoxia and venous plexus deoxygenation. By definition, primary livedo reticularis occurs in the absence of underlying disease and is ultimately a diagnosis of exclusion.

Idiopathic Livedo Reticularis: Idiopathic livedo reticularis differs from the previously two mentioned forms of livedo as the discoloration is permanent. Although the livedoid pattern may decrease with skin warming, it never completely resolves. Idiopathic livedo reticularis is also a diagnosis of exclusion, yet some authors believe it may represent an early, unrecognized manifestation of the antiphospholipid antibody syndrome or Sneddon's syndrome.

Risk Factors May Include:

Infections like tuberculosis, syphilis and Lyme's disease also may be associated.

Certain drugs may increase the chance of getting livedo reticularis.

Certain other conditions like antiphospholipid antibody syndrome affect coagulation of blood and cause formation of small blood clots in the arteries and veins.

Hypercalcemia.

Cryoglobulinaemia (a condition in which the blood contains large amounts of cryoglobulins which is a protein that is insoluble at low temperatures).

Arteriosclerosis.

Polycythemia rubra vera or thrombocytosis increase the risk of forming thrombi in the blood.

Symptoms:

Livedo reticularis causes a constriction or narrowing of the fine capillary blood vesselsthat feed the upper layers of the skin. In livedo reticularis, over time dilation of thesevessels causes the blood to stagnate, which causes a mottled discoloration of theoverlying skin. The rash in livedo reticularis is described as a reticular or lacy, net-likepurplish coloration surrounding a pale central area.The mottled appearance is related to spasms that occurin the dilated vessels. Mottling is more common in the forearms, thighs and lowerabdomen.Livedo reticularis occurs primarily on the legs, arms, and trunk with symptoms becomingmore pronounced in cold weather.

Diagnosis:

A complete history and physical examination should be performed with theintent of correctly classifying the patient as having either primary or secondarylivedo reticularis. The patient should be queried regarding amelioratingand exacerbating factors of their livedo (eg, effect of temperature andleg elevation) and whether or not any associated symptoms exist. With theexception of a subjective feeling of coldness, the majority of patients withlivedo reticularis are asymptomatic.

A detailed history forpotentially causative or exacerbating medications is essential (amantadine,vasoconstrictors, interferons) and one should investigate for coexistent systemicdiseases (autoimmune, hematologic, infectious, neurologic diseases)or interventional procedures (catheterization) known to be associated withlivedo reticularis. A history of venous thromboembolic disease and/or miscarriage maysuggest APAS (antiphospholipid antibody syndrome).

Sometime even skin biopsy is needed to rule out the underlying causes.

Treatment:

There is no exact cure for livedo reticularis. Other than cold avoidance, medical treatment for livedo reticularis without systemic association is typically unwarranted. A patient with primary livedo reticularis can benefit from episodic limb elevation. In rare instances, vasodilator therapy may be tried in the patient that is socially inhibited by the cosmetic appearance of the disorder, but there is no evidence-based medicine to support its use in this setting. Therapy of livedo reticularis with systemic association or livedo racemosa should be directed towards the underlying disorder (eg, immunosuppression for vasculitis, hydroxyurea for myeloproliferative syndromes, lowering calcium x phosphate product in calciphylaxis, etc).

Subjects with livedo racemosa and APAS complicated by arteriovenous thrombosis require anticoagulation. However, it is currently unknown how to manage the livedo racemosa patient with antiphospholipid antibodies and no history of arterial or venous thrombosis. No treatment is available that will effectively eliminate APAS-associated livedo racemosa, which can progress despite antiplatelet or anticoagulant therapy.

Treatment of livedoid vasculopathy is often unsatisfactory, but potentially beneficial medications include anticoagulants, antiplatelet agents, immunosuppressants, pentoxifylline, danazol, and tissue plasminogen activator. Alternatively, hyperbaric oxygen and psoralen plus UVA (PUVA) therapy have also been successfully utilized to treat livedoid vasculopathy. All patients with either primary or secondary forms of livedo are encouraged to avoid tobacco and the use of vasoconstricting medications.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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